Liver Histopathology in Pediatric Patients with Choledochal Cyst.

Aim: The aim is to study the various histopathological changes in the liver in pediatric patients with choledochal cyst (CC) and correlate with the presentation and type of cyst. Methods: In a prospective observational study including all pediatric patients who underwent CC excision, histopathological changes of the liver in the form of cholestasis (CHS), portal inflammation (PI), bile duct proliferation (BDP), and fibrosis were studied and graded using a scoring system. They were analyzed in relation to age, sex, symptoms, and type of the cyst. Results: All 30 patients of CC showed various degrees of histopathological changes in the liver in the form of CHS, PI, BDP, and liver fibrosis. Patients <1 years had 9/13 (69.2%) cystic variety and those >1 years had 17/17 (100%) fusiform variety of CC (P < 0.001). Patients <1 years frequently presented with jaundice and hepatomegaly and those >1 years presented with pain abdomen (P < 0.002). Higher grades of liver fibrosis and BDP were seen in the cystic variety compared to the fusiform variety (P < 0.001). However, no significant association was found with CHS and PI (P > 1.23). Conclusions: Histopathological changes in the liver of varying grades are seen in all patients of CC. Patients of CC <1 year presented frequently with jaundice, had the cystic type, and had a higher degree of liver damage on histopathology.

Evaluation of the Outcome of Pyeloplasty in Children with Poorly Functioning Kidneys due to Unilateral Ureteropelvic Junction Obstruction.

Aim: To analyze the outcome of upfront pyeloplasty in kidneys of children with unilateral ureteropelvic junction obstruction (UPJO). Materials and Methods: Thirty-three consecutive cases with split renal function (SRF) of ≤20% on dynamic renal scintigraphy (DRS) underwent upfront pyeloplasty with a nephrostomy tube and trans-anastomotic stent. Outcome was analyzed based on symptomatic relief, nephrostomy output, surgical complications and changes noted in pre-and post-operative findings on renal ultrasound (US), and DRS. Results: The most common symptom was abdominal lump in <5-year age group (79%) and abdominal pain in >5-year age group (93%). Postoperatively, symptoms were relieved in all (100%), parenchymal thickness (PT) on US improved in 82% and SRF improved significantly (>5%) in 75.8% of patients. The improvement was more significant in patients with abdominal lump and large kidneys. The mean nephrostomy output showed an inverse relationship with age at pyeloplasty and a direct correlation with the change in PT and SRF. The degree of improvement in SRF also was inversely related to the age at pyeloplasty with a significantly better outcome in <2-year-age. Although age at pyeloplasty, nephrostomy output and change in PT individually showed significant correlation with change in SRF, multiple regression analysis showed PT as the only significant factor. Conclusion: Upfront pyeloplasty should be the first option in children with poorly functioning kidneys as it has a favorable outcome in almost all the cases with a very low incidence of complications. The degree of improvement in SRF can be predicted by the nephrostomy output and improvement in PT on US.

Morbidity and Mortality of Necrotizing Fasciitis and Their Prognostic Factors in Children.

Background: This is a prospective study of the clinico-etiologic profile and factors affecting outcomes in 40 children managed for necrotizing fasciitis (NF). Materials and Methods: Demographic details, clinical characteristics, and laboratory parameters were recorded, and the Laboratory Risk Indicator for Necrotizing Fasciitis (LRINEC) score was calculated. Primary outcome (survival vs. nonsurvival) was noted, and prognostic factors were identified. Results: Initiating factors included boils (45%), i.v. cannula extravasations (22.5%), and blunt trauma (17.5%). Lesion (s) were predominantly on the lower limbs (35%) and trunk (25%). Twenty-two patients (55%) had <5% body surface area (BSA) involved. Severely deranged clinical and laboratory parameters were common. Ultrasound localized fluid collections. Pus cultures showed methicillin-resistant Staphylococcus aureus (52.5%), methicillin-sensitive S. aureus [27.5%], and polymicrobial growth (20%). Blood culture was positive in 24 patients (60%). Most isolates were sensitive to clindamycin and amoxy-clavulanate. Prognostic factors for mortality (n = 6; 15%) included categorization as "Sick," BSA involvement >10%, thrombocytopenia, raised serum creatinine, late debridement, and polymicrobial blood culture isolates. All six nonsurvivors had a LRINEC score of ≥8 and positive blood cultures. Six patients (20.7%) developed unsightly scars and 5 (17.24%) contractures across joints. Conclusions: Pediatric NF has significant morbidity and mortality. Patients with adverse prognostic factors can benefit from early referral to a facility with a critical care unit. Adequate wound management is essential to minimize residual deformity.

Influence of Parental Awareness Drive on Preoperative Fasting Compliance in Pediatric Day Care Surgery.

Context: Optimum preoperative fasting is imperative for the prevention of aspiration in pediatric patients. The current guidelines advocate 2-4-6 rule for the same. However, direct supervision is lacking in large volume centers. Aims: Thus, we aimed to determine the fasting compliance of children preoperatively and to ascertain whether parents understood the significance and purpose of optimum fasting. Materials and Methods: Design - A prospective questionnaire-based study regarding preoperative fasting was performed in pediatric patients aged 1-10 years scheduled for "day care surgery" or "same day admission surgery" over 12 weeks. Thereafter, parental awareness drive was carried out, and a re-audit was performed with a questionnaire in the next cohort of patients. Results: The number of patients in the pre and postcounseling groups were 98 and 99. Thirteen percent of the patients were optimally fasted for solids initially. Re-audit confirmed compliance increased to 46%. Patients fasting adequately (2-3 h) for clear fluids increased from 22.4% to 51.5% postcounseling. The number for optimally breast-fed children increased postaudit (23.1%-39.1%). Consequent to the drive, parental awareness increased and 49.5% parents knew that only plain water was permitted during fasting. Number of parents considering preoperative fasting important increased from 39.8% to 79.7%. Initially, 27.6% of the parents did not know the reason for fasting, which reduced to 3% postaudit. Conclusion: Parents are misinformed and ignorant about optimum preoperative fasting. Adequate education and awareness to improve their knowledge was associated with increased compliance for optimal fasting.

Correlation of the Position of the Anal Dimple with the Caudal Termination of the Striated Muscle Complex in Patients with Anorectal Malformations.

Background: In children with anorectal malformations (ARM), the vertical fibres of the striated muscle complex (SMC) are believed to be located within the limits of the anal dimple (AD). Methods: Forty five cases of ARM underwent posterior sagittal anorectoplasty (PSARP), median age 8.5 months. During PSARP the anterior, posterior limits and midpoint of the AD and SMC were marked. The location of AD was correlated with SMC. Results: A 'well developed' AD and SMC was seen in 80 % and 86.7 % patients respectively. The mean width of the AD and SMC was more in females than in males (20.96 vs. 18.98 mm and 14.24mm vs. 13.45mm respectively). In 36 cases (80 %), across the spectrum of ARM, the SMC was posterior in relation to the AD. In 7 cases (15.5 %) it correlated in position with the AD and in 2 cases (4.44 %), it was anterior to the AD. Conclusions: In the majority of cases AD and SMC were 'well developed' and the location of the SMC does not correlate with that of the AD. This has significant practical value in the important step of optimizing the placement of the rectum through the center of the SMC during repair.

Congenital Pouch Colon in Girls: An Algorithm for Preoperative Diagnosis.

Aim: The aim of the study was o define the preoperative diagnostic clinical and radiological features in girls with congenital pouch colon (CPC). Materials and Methods: Records of 47 girls with CPC, managed from 1996 to 2018, were reviewed. There were two age groups: Group A (newborn to 12 months; n = 26) and Group B (>12 months to 20 years; n = 21). The important clinical and radiologic features to help in a preoperative diagnosis were noted. Results: The most common subtype was Type II (57.4%), followed by Type I (23.4%) and Type III (12.8%). The features common to both the groups were abdominal distension (A = 53.8%; B = 9.52%), severe perineal excoriation (A = 19.2%; B = 23/8%), and urinary incontinence (A = 30.7%; B = 85.7%). In addition, in Group B, fecaloma on abdominal palpation was noted in 28.6% of patients. The characteristic appearance of the perineum including external genitalia and findings on plain abdominal X-ray (AXR) were 100% accurate and hence diagnostic. These unique features helped us formulate an algorithm for preoperative diagnosis of this uncommon form of anorectal malformation in girls seen in North India. Conclusions: The characteristic features on clinical examination should alert one to the presence of CPC in the outpatient clinic. The AXR was diagnostic in 100% of cases and is mandatory. If any doubt persists, examination of the genitalia under anesthesia with more retraction of the labial folds and endoscopy can be performed for confirmation. These measures should enable a clinician to make an accurate preoperative diagnosis in every girl with CPC.

Magnitude of SARS-CoV-2 Infection and Outcome in Paediatric Surgical Inpatients during the First Wave of Pandemic at a Tertiary Care Children's Hospital in India.

This study was conducted at a tertiary care centre of Delhi, to assess the magnitude of SARS-CoV-2 disease and outcome in paediatric surgical inpatients. All the admitted patients were evaluated excluding outpatients and minor procedures. Out of 312 operated patients 2.88% patients were SARS-CoV-2 positive and out of 167 non-operative patients 6.58% were SARS-CoV-2 positive. These patients received standard care as per guidelines using standard protective measures and were discharged home. Only 1 death occurred due to perforation peritonitis with sepsis. The overall prevalence of SARS-CoV-2 in paediatric surgical patients was 4.17% and SARS-CoV-2 positive patients had similar outcomes as compared to non-SARS-CoV-2 patients.

Results of Injection Sclerotherapy with Bleomycin in Pediatric Lymphatic Malformations.

AIM: The aim of the study was to evaluate the results of injection sclerotherapy with bleomycin in pediatric patients with lymphatic malformations. MATERIALS AND METHODS: In this prospective cohort study, all consenting pediatric patients with macrocystic lymphatic malformations were managed with injection bleomycin sclerotherapy (0.5 mg/kg, not exceeding 5 mg at a time) under ultrasound (US) guidance. After aspirating the cyst fluid bleomycin was instilled intralesionally in a ratio of 5:1 (aspirated cyst fluid volume: diluted bleomycin solution volume). Patients were reassessed at three weekly intervals. The response to therapy was assessed clinically as well as by size and volume on ultrasound Doppler study. The response was classified as excellent response, i.e., complete regression, good response >50% regression, and poor response <50% regression. RESULTS: Sixty patients with lymphatic malformations were enrolled in the study, the mean age was 3.22 years, and the male-to-female was 2.5:1. The most common site of lesion was in the neck (43.3%), followed by the axilla (15%) and flank (8.3%). The responses were excellent, good, and poor in 43 (71.6%), 12 (20%), and five (8.3%) patients, respectively. Two patients underwent surgical excision of the residual lesion. Complications noted were fever in six, local pain in five, and residual lesion in three patients. CONCLUSION: Sclerotherapy with bleomycin is simple, safe, and effective in the first line of management for macrocystic lymphatic malformations in children.

A Step-by-Step Sonographic Approach to Vascular Anomalies in the Pediatric Population: A Pictorial Essay.

Vascular anomalies are a common cause of soft-tissue masses in children and often referred for ultrasonographic (USG) evaluation. They are broadly classified as vascular tumors (hemangiomas, hemangioendotheliomas, and angiosarcomas) or vascular malformations (venous malformations, lymphatic malformations, and arteriovenous malformations). Findings on USG and Doppler imaging can be used to categorize vascular anomalies into high- or low-flow lesions, which forms the basis for further workup, diagnosis, and management. On careful evaluation of various sonographic features, in conjunction with clinical findings, an accurate clinicoradiological diagnosis can be made in most cases. Further imaging with magnetic resonance (MR) imaging or computed tomography (CT) helps in delineation of lesion extent, whereas MR or CT angiography is useful to map the vascular supply of high-flow lesions. We have illustrated and discussed a step-by-step approach to diagnose vascular anomalies using ultrasound and Doppler imaging.

An Analysis of Safety and Efficacy of Day-care Surgery in Children in a Tertiary Care Hospital in India.

BACKGROUND: Advances in surgery and anesthesia have paved the way for the establishment of day-care surgery (DCS). Observations that children achieve better convalescence in the home environment along with significant economic advantages have led to this paradigm shift in clinical practice. AIMS AND OBJECTIVES: This study is aimed to evaluate the feasibility of performing various surgical procedures on day-care basis and assess parental satisfaction with DCS in children. MATERIALS AND METHODS: In this prospective observational study, all children >3 months of age undergoing various elective surgical procedures as day-care cases in our institution were enrolled. Types of operations, complications, including any unplanned admissions and parental satisfaction, were recorded. RESULTS: Between December 2015 and December 2018, a total of 654 day-care surgeries were performed in our institution by pediatric surgeons. The mean age was 5.5 years with M: F 5.5:1. Thirty different surgical procedures were successfully performed as DCS, the common procedures being inguinal herniotomy (31.5%), and orchidopexy (14.3%). Unplanned admissions were recorded in 2.29% (15/654) patients (scrotal edema-5, postoperative pain-8, and a long recovery from anesthesia-2). No major complications occurred; two minor complications during follow-up were superficial wound infection and drug reaction. Overall parental satisfaction was very high (100%)-preoperative prolonged fasting period and long waiting time in the preoperative room of afternoon shift patients (7.95% and 8.3%) were the reasons for their discontent. CONCLUSIONS: DCS in children is safe and effective with high parental satisfaction. It can substantially reduce the waiting list for several surgical procedures in children.

Evolving Concepts in Ultrasonography of Pediatric Intussusceptions: Unequivocal Differentiation of Ileocolic, Obstructive and Transient Small-Bowel Intussusceptions.

The main aim of the study was to elaborate on the various ultrasonographic parameters that can be used for definite differentiation of ileocolic, obstructive small-bowel and transient small-bowel intussusceptions (SBI). In this study, 90 children (63 boys and 27 girls) with suspected intussusception were evaluated. Of these, 54 cases were diagnosed as obstructive intussusceptions (33 ileocolic and 21 ileoileal), 15 cases were classified as transient SBIs and 21 cases were negative for intussusception. The mean fat core diameter was 1.45 ± 0.32 cm for ileocolic versus 0.37 ± 0.06 cm for obstructive SBI versus 0.29 ± 0.08 cm for transient SBI (p < 0.001). The mean lesion diameter was 3.23 ± 0.08 cm for ileocolic intussusceptions and 2.12 ± 0.038 cm for SBI (p < 0.001), and the ratio of inner fat core to outer wall thickness was greater than 1 for ileocolic intussusceptions and less than 1 for SBI. A statistically significant difference was found between segmental invagination of transient versus obstructive SBIs with mean values of 1.93 ± 0.39 cm and 3.17 ± 0.25 cm, respectively, and an "optimal" threshold at 2.5 cm.

Prediction of Gap Length by Plain Radiograph of Chest with Nasogastric Tube in the Upper Esophagus in Patients with Esophageal Atresia and Distal Tracheoesophageal Fistula.

AIM: This study was aimed at prediction of the gap length between the two esophageal ends in cases of esophageal atresia and distal tracheoesophageal fistula (EA-TEF) by preoperative radiography with a nasogastric tube in the upper esophagus and its correlation with intraoperatively measured gap length. MATERIALS AND METHODS: All consecutive cases of EA-TEF were prospectively included in this study. Plain radiographs were taken with an 8 Fr nasogastric tube inserted in the upper esophageal pouch till its arrest. The patients were grouped into T1-T2; T2-T3; T3-T4; and T4 groups depending on the thoracic vertebral level of arrest of the NG tube on the radiograph. Intraoperative gap between the two esophageal ends was measured with Vernier caliper, and the patients were grouped into A, B, and C groups based on gap length (gap length >2.1 cm; >1-≤2 cm; and ≤1 cm). The operative gap groups were compared with the radiography groups. RESULTS: A total number of 118 cases were included over a period of 3 years. The arrest of nasogastric tube at T1-T2 and T2-T3 vertebral level corresponded to gap length Group A in 39/41 (95.12%) * patients. In gap length Group B, the arrest of tube at T2-T3 and T3-T4 vertebral level was seen in 44/44 (100%)* patients, in gap length Group C, the arrest of tube was noted at T3-T4 and T4 vertebral level in 31/33 (93.93%)* patients (*P < 0.001). CONCLUSION: Prediction of gap length by vertebral level of arrest of the nasogastric tube in the upper pouch in a preoperative chest X-ray correlated well with intra operatively measured gap length in cases of EA-TEF.

Radiologic Evaluation of Uterovaginal Abnormalities in Girls with Congenital Pouch Colon.

BACKGROUND/PURPOSE: The radiologic investigations of 25 girls with congenital pouch colon (CPC), managed over 17 years, were retrospectively reviewed. In 13 girls who form the study group, the investigations provided information about the anomalous uterovaginal (UV) anatomy and these findings were studied. MATERIALS AND METHODS: Age at presentation was 2 months to 10 years. The subtypes of CPC were Type I (n = 2), Type II (n = 9), and not recorded (n = 2). All patients had a double vagina and a unicornuate uterus on each side in the pelvis. The radiologic studies, performed at varying periods after surgery, included an intravenous urogram (IVU) (n = 4), micturating cystourethrogram (MCU) (n = 3), distal ileostogram/colostogram (n = 6), and magnetic resonance imaging (MRI) (n = 7). RESULTS: IVU and MCU showed retrograde filling of the vaginas with contrast during micturition with a small-capacity urinary bladder and a relatively open bladder neck. The two vaginas were quite apart, but symmetrical in appearance and position. A distal dye study showed filling of the colonic pouch, its terminal fistula, and the two vaginas in six patients. Opacification of the bladder was seen in 3/6 girls, including one girl with left-sided Grade IV vesicoureteral reflux. MRI (n = 7) showed a monocornuate uterus on each side in the pelvis. The upper vaginas on each side were apart, being widely apart (n = 5) and somewhat closer (n = 2). The lower vaginas were closer with an intervaginal septum. Other findings were a widely open bladder neck and urethra in two girls with urinary incontinence and visualization of the terminal fistula of the colonic pouch (n = 2). The lumbosacral spine was normal in all patients. CONCLUSIONS: In girls with CPC, retrograde reflux of contrast into the vaginas during a distal dye study or an IVU/MCU may provide useful details of the anatomy of the vaginas. An MRI scan is recommended as essential for comprehensive evaluation of the anomalous UV anatomy.

Anorectal Agenesis with Rectovaginal Fistula: A Rare/Regional Variant.

AIMS AND OBJECTIVES: The anatomical types of female anorectal malformation (ARM) are complex and include several rare and regional variants. The aim of this report is to highlight the diagnosis and management of cases of anorectal agenesis with rectovaginal fistula (RVF). MATERIALS AND METHODS: This is a retrospective chart review of cases of RVF who underwent definitive surgery between November 2000 and September 2016 in a single institution. RESULTS: Of the 244 female patients with anorectal anomalies treated in our institution over a 16-year period, there were 15 cases of RVF with anorectal agenesis giving it an incidence of 6%. All cases of RVF with anorectal agenesis presented with absence of anal opening, varying degree of abdominal distension, and history of passage of fecal matter per vaginum. The mean age of presentation was 3 months (newborn to 1 year). Sigmoid colostomy was done in 13 cases. Distal colostogram demonstrated high RVF which was further confirmed by examination under anesthesia (EUA) and endoscopy before definitive surgery. With the posterior sagittal approach, division of the RVF and anorectoplasty was possible in 14 cases; however, one patient required an abdominal mobilization of the colon for pull through due to a very short distal bowel segment. During a mean postoperative follow-up of 5 years, cosmetic and functional results were satisfactory. CONCLUSION: RVF with anorectal agenesis is a rare/regional variant of female ARMs. Clinical examination along with distal colostogram, EUA, and endoscopy clinches the diagnosis. Anorectal reconstruction by posterior sagittal anorectoplasty results in a satisfactory outcome.

Assessment of Nutritional Status of Patients of Congenital Pouch Colon Following Definitive Surgery.

PURPOSE: To assess the nutritional status in 31 patients of congenital pouch colon (CPC) who had undergone definitive surgery and closure of a protective stoma, if any, at least 1 year earlier and were below 14 years age. MATERIALS AND METHODS: The clinical history, demographic details, anthropometric measurements, and results of hematological and biochemical tests were recorded. In addition to collective data, analysis was also performed after grouping by age, subtype of CPC (Types I/II and Types III/IV CPC), and in Types I/II CPC patients, by whether the colonic pouch had been completely excised or else a segment preserved by tubular colorraphy (TC). RESULTS: Severe fecal incontinence (FI) was common (64.52%). Anthropometry showed a significant malnutrition in 53.85-95.45% patients, especially stunting which was most prevalent in the 0-5 years age-group. Serum Vitamin B12, folate, and Vitamin D were lower than normal in 38.71%, 22.58%, and 74.19% patients, respectively, without statistically significant difference among the various groups studied. Patients with Types I/II CPC had a statistically significant higher incidence of anemia, low serum ferritin, and severe FI than patients with Types III/IV CPC. Patients with Types I/II CPC, managed by excision of the colonic pouch, had a higher incidence of severe FI, wasting, and thinness than those undergoing TC. CONCLUSIONS: On follow-up of the patients of CPC, anthropometry shows a high incidence of malnutrition, especially stunting in the 0-5 years age-group. There is an adequate adaptation of fluid-electrolyte homeostasis. Although Types I/II CPC patients have a significantly higher incidence of anemia and severe FI than Types III/IV CPC patients, long-term anthropometric parameters are similar. In Types I/II CPC, preservation of the colonic pouch by TC offers long-term benefit.

Pediatric esophageal substitution by gastric pull-up and gastric tube.

AIM: The aim of this study was to report the results of pediatric esophageal substitution by gastric pull-up (GPU) and gastric tube (GT) from a tertiary care pediatric center. MATERIALS AND METHODS: Retrospective analysis of the surgical techniques, results, complications, and final outcome of all pediatric patients who underwent esophageal substitution in a single institution was performed. RESULTS: Twenty-four esophageal substitutions were performed over 15-year period. The indications were pure esophageal atresia (EA)-19, EA with distal trachea-esophageal fistula-2, EA with proximal pouch fistula-1, and esophageal stricture in two patients. Mean age and weight at operation were 17 months and 9.5 kg, respectively. GPU was the most common procedure (19) followed by reverse GT (4) and gastric fundal tube (1). Posterior mediastinal and retrosternal routes were used in 17 and 7 cases, respectively. Major complications included three deaths in GPU cases resulting from postoperative tachyarrhythmias leading to cardiac arrest, cervical anastomotic leak-17, and anastomotic stricture in six cases. Perioperative tachyarrhythmias (10/19) and transient hypertension (2/19) were observed in GPU patients, and they were managed with beta blocker drugs. Postoperative ventilation in Intensive Care Unit was performed for all GPU, but none of the GT patients. Follow-up ranged from 6 months to 15 years that showed short-term feeding difficulties and no major growth-related problems. CONCLUSIONS: Perioperative tachyarrhythmias are common following GPU which mandates close intensive care monitoring with ventilation and judicious use of beta blocking drugs. Retrosternal GT with a staged neck anastomosis can be performed without postoperative ventilation.

Determinants of gap length in esophageal atresia with tracheoesophageal fistula and the impact of gap length on outcome.

AIM: This study was aimed at identifying factors which may affect the gap length in cases of esophageal atresia with tracheoesophageal fistula (EA-TEF) and whether gap length plays any role in determining the outcome. MATERIALS AND METHODS: All consecutive cases of EA-TEF were included and different patient parameters were recorded. Plain radiographs with a nasogastric tube in the upper esophagus were taken. Patients were grouped into T1-T2; T2-T3; T3-T4; and T4 depending on the thoracic vertebral level of the arrest of the tube. During surgery, the gap length between the pouches was measured using a Vernier caliper and the patients were grouped into A, B, and C (gap length >2.1 cm; >1-≤2 cm and ≤1 cm). The operative gap groups were compared with the radiography groups and the other recorded parameters. RESULTS: Total numbers of cases were 69. Birth weight was found to be significantly lower in Group A (mean = 2.14 kg) as compared to Group B (mean = 2.38 kg) and Group C patients (mean = 2.49 kg) (P = 0.016). The radiographic groups compared favorably with the intraoperative gap length groups (P < 0.001). The need for postoperative ventilation (70.83% in Group A vs. 36.84% in Group C, P = 0.032) and mortality (62.5%, 26.9% and 15.8% in Group A, B, and C, respectively, P = 0.003) co-related significantly with the gap length. CONCLUSION: Birth weight had a direct reciprocal relationship with the gap length. Radiographic assessment correlated with intraoperative gap length. Higher gap length was associated with increased need for postoperative ventilation and poor outcome.

Congenital pouch colon in girls: Genitourinary abnormalities and their management.

AIMS: To discuss the assessment and management of genitourinary (GU) tract abnormalities in 21 girls with Types I-III congenital pouch colon (CPC), studied over a period of 10 years. MATERIALS AND METHODS: Assessment included clinical and radiological assessment, examination under anesthesia (EUA), endoscopy of the lower GU tract, and evaluation of the surgical findings, operative procedures for the GU anomalies, and the results of management. RESULTS: Initial examination of the external genitalia showed a "clover-leaf" appearance (n = 6) and a single perineal opening (n = 6). In 9 patients, the openings of the urethra and double vagina were seen, of which a vestibular fistula was seen in 5 and an anterior perineal fistula in 1. Seventeen patients (81%) had urinary incontinence (UI) - partial in 10, and complete in 7. Renal function tests, X-ray sacrum, and abdominal US were normal in all patients. Micturating cystourethrogram (n = 9) showed a wide, bladder neck incompetence (BNI) with reduced bladder capacity in seven patients. EUA and endoscopy revealed a septate vagina in all patients and the urethral opening at a "high" position (n = 14) or at a relatively normal or "low" position (n = 7). In 8 patients, the intervaginal septum was thick and fleshy. Endoscopy showed a short, wide urethra, an open incompetent bladder neck, poorly developed trigone, and reduced bladder capacity in the patients with UI. The fistula from the colonic pouch opened in the proximal urethra (n = 4), high in the vestibule (n = 3), low in the vestibule (n = 8), perineum just posterior to the vestibule (n = 1), and undetermined (n = 5). Vaginoscopy (n = 8) showed normal cervices in all and cervical mucus in 4 patients. The subtypes of CPC were Type I CPC (n = 4), Type II CPC (n = 16), and Type III CPC (n = 1). All 21 patients had uterus didelphys. In four patients with UI, during tubular colorraphy, a segment of the colonic pouch was preserved for later bladder augmentation if required. A Young-Dees bladder-neck repair (BNR) was performed in four older girls for treatment of UI, with marked improvement in urinary continence in two girls, some improvement in one girl with complete urinary incontinence, and minimal improvement in one child. Division of the intervaginal septum was performed in three girls. CONCLUSIONS: GU abnormalities in girls with CPC need to be assessed and managed by a tailored protocol. UI is frequent, and its correction may require BNR. A segment of the colonic pouch can be preserved for possible future bladder augmentation. All girls have a septate vagina, often widely separated, and uterus didelphys. Gynecologic assessment and monitoring is required throughout adult life. Considering the wide opening of the vestibule, surgical management of the urogenital component by division of the intervaginal septum and if required, the vagino-fistula septum on each side results in a normal or a hypospadiac urethral opening and an adequate vaginal inlet.